Eculizumab in STEC-HUS: need for a proper randomized controlled trial

go back to reference Tarr PI, Gordon CA, Chandler WL (2005) Shiga-toxin-producing Escherichia coli and haemolytic uraemic syndrome. Lancet 365:1073–1086PubMed Tarr PI, Gordon CA, Chandler WL (2005) Shiga-toxin-producing Escherichia coli and haemolytic uraemic syndrome. Lancet 365:1073–1086PubMed

go back to reference Obrig TG, Karpman D (2012) Shiga toxin pathogenesis: kidney complications and renal failure. Curr Top Microbiol Immunol 357:105–136PubMedPubMedCentral Obrig TG, Karpman D (2012) Shiga toxin pathogenesis: kidney complications and renal failure. Curr Top Microbiol Immunol 357:105–136PubMedPubMedCentral

go back to reference Nester CM, Barbour T, Rodriquez de Cordoba S, Dragon-Durey MA, Fremeaux-Bacchi V, Goodship THJ, Kavanagh D, Noris M, Pickering M, Sanchez-Corral P, Skerka C, Zipfel P, Smith RJH (2015) Atypical aHUS: state of the art. Mol Immunol 67:31–42CrossRefPubMed Nester CM, Barbour T, Rodriquez de Cordoba S, Dragon-Durey MA, Fremeaux-Bacchi V, Goodship THJ, Kavanagh D, Noris M, Pickering M, Sanchez-Corral P, Skerka C, Zipfel P, Smith RJH (2015) Atypical aHUS: state of the art. Mol Immunol 67:31–42CrossRefPubMed

go back to reference Loirat C, Saland J, Bitzan M (2012) Management of hemolytic uremic syndrome. Presse Med 41:e115–e135CrossRefPubMed Loirat C, Saland J, Bitzan M (2012) Management of hemolytic uremic syndrome. Presse Med 41:e115–e135CrossRefPubMed

go back to reference Thurman JM, Marians R, Emlen W, Wood S, Smith C, Akana H, Holers VM, Lesser M, Kline M, Hoffman C, Christen E, Trachtman H (2009) Alternative pathway of complement in children with diarrhea-associated hemolytic uremic syndrome. Clin J Am Soc Nephrol 4:1920–1924CrossRefPubMedPubMedCentral Thurman JM, Marians R, Emlen W, Wood S, Smith C, Akana H, Holers VM, Lesser M, Kline M, Hoffman C, Christen E, Trachtman H (2009) Alternative pathway of complement in children with diarrhea-associated hemolytic uremic syndrome. Clin J Am Soc Nephrol 4:1920–1924CrossRefPubMedPubMedCentral

go back to reference Ferraris JR, Ferraris V, Acquier AB, Sorroche PB, Saez MS, Ginaca A, Mendez CF (2015) Activation of the alternative pathway of complement during the acute phase of typical haemolytic uraemic syndrome. Clin Exp Immunol 181:118–125CrossRefPubMedPubMedCentral Ferraris JR, Ferraris V, Acquier AB, Sorroche PB, Saez MS, Ginaca A, Mendez CF (2015) Activation of the alternative pathway of complement during the acute phase of typical haemolytic uraemic syndrome. Clin Exp Immunol 181:118–125CrossRefPubMedPubMedCentral

go back to reference Monnens L, Molenaar J, Lambert PH, Proesmans W, van Munster P (1980) The complement system in hemolytic-uremic syndrome in childhood. Clin Nephrol 13:168–171PubMed Monnens L, Molenaar J, Lambert PH, Proesmans W, van Munster P (1980) The complement system in hemolytic-uremic syndrome in childhood. Clin Nephrol 13:168–171PubMed

go back to reference Ahlenstiel-Grunow T, Hachmeister S, Bange FC, Wehling C, Kirschfink M, Bergmann C, Pape L (2016) Systemic complement activation and complement gene analysis in enterohaemorrhagic Escherichia coli-associated paediatric haemolytic uraemic syndrome. Nephrol Dial Transplant 31:1114–1121CrossRefPubMed Ahlenstiel-Grunow T, Hachmeister S, Bange FC, Wehling C, Kirschfink M, Bergmann C, Pape L (2016) Systemic complement activation and complement gene analysis in enterohaemorrhagic Escherichia coli-associated paediatric haemolytic uraemic syndrome. Nephrol Dial Transplant 31:1114–1121CrossRefPubMed

go back to reference Morigi M, Galbusera M, Gastoldi S, Locatelli M, Buelli S, Pezzotta A, Pagani C, Noris M, Gobbi M, Stravalaci M, Rottoli D, Tedesco F, Remuzzi G, Zoja C (2011) Alternative pathway activation of complement by Shiga toxin promotes exuberant C3a formation that triggers microvascular thrombosis. J Immunol 187:172–180CrossRefPubMed Morigi M, Galbusera M, Gastoldi S, Locatelli M, Buelli S, Pezzotta A, Pagani C, Noris M, Gobbi M, Stravalaci M, Rottoli D, Tedesco F, Remuzzi G, Zoja C (2011) Alternative pathway activation of complement by Shiga toxin promotes exuberant C3a formation that triggers microvascular thrombosis. J Immunol 187:172–180CrossRefPubMed

go back to reference Orth-Holler D, Wurzner R (2014) Role of complement in enterohemorrhagic Escherichia coli-induced hemolytic uremic syndrome. Semin Thromb Hemost 40:503–507CrossRefPubMed Orth-Holler D, Wurzner R (2014) Role of complement in enterohemorrhagic Escherichia coli-induced hemolytic uremic syndrome. Semin Thromb Hemost 40:503–507CrossRefPubMed

go back to reference Arvidsson I, Rebetz J, Loos S, Herthelius M, Kristoffersson AC, Englund E, Chromek M, Karpman D (2016) Early terminal complement blockade and C6 deficiency are protective in Enterohemorrhagic Escherichia coli-infected mice. J Immunol 197:1276–1286CrossRefPubMed Arvidsson I, Rebetz J, Loos S, Herthelius M, Kristoffersson AC, Englund E, Chromek M, Karpman D (2016) Early terminal complement blockade and C6 deficiency are protective in Enterohemorrhagic Escherichia coli-infected mice. J Immunol 197:1276–1286CrossRefPubMed

go back to reference Westra D, Volokhina EB, van der Molen RG, van der Velden TJ, Jeronimus-Klaasen A, Goertz J, Gracchi V, Dorresteijn EM, Bouts AH, Keijzer-Veen MG, van Wijk JA, Bakker JA, Roos A, van den Heuvel LP, van de Kar NC (2017) Serological and genetic complement alterations in infection-induced and complement-mediated hemolytic uremic syndrome. Pediatr Nephrol 32:297–309CrossRefPubMed Westra D, Volokhina EB, van der Molen RG, van der Velden TJ, Jeronimus-Klaasen A, Goertz J, Gracchi V, Dorresteijn EM, Bouts AH, Keijzer-Veen MG, van Wijk JA, Bakker JA, Roos A, van den Heuvel LP, van de Kar NC (2017) Serological and genetic complement alterations in infection-induced and complement-mediated hemolytic uremic syndrome. Pediatr Nephrol 32:297–309CrossRefPubMed

go back to reference Zoja C, Buelli S, Morigi M (2017) Shiga toxin triggers endothelial and podocyte injury: the role of complement activation. Pediatr Nephrol. https://doi.org/10.1007/s00467-017-3850-x Zoja C, Buelli S, Morigi M (2017) Shiga toxin triggers endothelial and podocyte injury: the role of complement activation. Pediatr Nephrol. https://​doi.​org/​10.​1007/​s00467-017-3850-x

go back to reference Adamski J (2014) Thrombotic microangiopathy and indications for therapeutic plasma exchange. Hematology Am Soc Hematol Educ Program 2014:444–449PubMed Adamski J (2014) Thrombotic microangiopathy and indications for therapeutic plasma exchange. Hematology Am Soc Hematol Educ Program 2014:444–449PubMed

go back to reference Michael M, Elliott EJ, Craig JC, Ridley G, Hodson EM (2009) Interventions for hemolytic uremic syndrome and thrombotic thrombocytopenic purpura: a systematic review of randomized controlled trials. Am J Kidney Dis 53:259–272CrossRefPubMed Michael M, Elliott EJ, Craig JC, Ridley G, Hodson EM (2009) Interventions for hemolytic uremic syndrome and thrombotic thrombocytopenic purpura: a systematic review of randomized controlled trials. Am J Kidney Dis 53:259–272CrossRefPubMed

go back to reference Keenswijk W, Raes A, Vande Walle J (2018) Is eculizumab efficacious in Shigatoxin-associated hemolytic uremic syndrome? A narrative review of current evidence. Eur J Pediatr 177:311–318CrossRefPubMed Keenswijk W, Raes A, Vande Walle J (2018) Is eculizumab efficacious in Shigatoxin-associated hemolytic uremic syndrome? A narrative review of current evidence. Eur J Pediatr 177:311–318CrossRefPubMed

go back to reference Spinale JM, Ruebner RL, Copelovitch L, Kaplan BS (2013) Long-term outcomes of Shiga toxin hemolytic uremic syndrome. Pediatr Nephrol 28:2097–2105CrossRefPubMed Spinale JM, Ruebner RL, Copelovitch L, Kaplan BS (2013) Long-term outcomes of Shiga toxin hemolytic uremic syndrome. Pediatr Nephrol 28:2097–2105CrossRefPubMed

go back to reference Lapeyraque AL, Malina M, Fremeaux-Bacchi V, Boppel T, Kirschfink M, Oualha M, Proulx F, Clermont MJ, Le Deist F, Niaudet P, Schaefer F (2011) Eculizumab in severe Shiga-toxin-associated HUS. N Engl J Med 364:2561–2563CrossRefPubMed Lapeyraque AL, Malina M, Fremeaux-Bacchi V, Boppel T, Kirschfink M, Oualha M, Proulx F, Clermont MJ, Le Deist F, Niaudet P, Schaefer F (2011) Eculizumab in severe Shiga-toxin-associated HUS. N Engl J Med 364:2561–2563CrossRefPubMed

go back to reference Menne J, Nitschke M, Stingele R, Abu-Tair M, Beneke J, Bramstedt J, Bremer JP, Brunkhorst R, Busch V, Dengler R, Deuschl G, Fellermann K, Fickenscher H, Gerigk C, Goettsche A, Greeve J, Hafer C, Hagenmuller F, Haller H, Herget-Rosenthal S, Hertenstein B, Hofmann C, Lang M, Kielstein JT, Klostermeier UC, Knobloch J, Kuehbacher M, Kunzendorf U, Lehnert H, Manns MP, Menne TF, Meyer TN, Michael C, Munte T, Neumann-Grutzeck C, Nuernberger J, Pavenstaedt H, Ramazan L, Renders L, Repenthin J, Ries W, Rohr A, Rump LC, Samuelsson O, Sayk F, Schmidt BM, Schnatter S, Schocklmann H, Schreiber S, von Seydewitz CU, Steinhoff J, Stracke S, Suerbaum S, van de Loo A, Vischedyk M, Weissenborn K, Wellhoner P, Wiesner M, Zeissig S, Buning J, Schiffer M, Kuehbacher T (2012) Validation of treatment strategies for enterohaemorrhagic Escherichia coli O104:H4 induced haemolytic uraemic syndrome: case-control study. BMJ 345:e4565CrossRefPubMedPubMedCentral Menne J, Nitschke M, Stingele R, Abu-Tair M, Beneke J, Bramstedt J, Bremer JP, Brunkhorst R, Busch V, Dengler R, Deuschl G, Fellermann K, Fickenscher H, Gerigk C, Goettsche A, Greeve J, Hafer C, Hagenmuller F, Haller H, Herget-Rosenthal S, Hertenstein B, Hofmann C, Lang M, Kielstein JT, Klostermeier UC, Knobloch J, Kuehbacher M, Kunzendorf U, Lehnert H, Manns MP, Menne TF, Meyer TN, Michael C, Munte T, Neumann-Grutzeck C, Nuernberger J, Pavenstaedt H, Ramazan L, Renders L, Repenthin J, Ries W, Rohr A, Rump LC, Samuelsson O, Sayk F, Schmidt BM, Schnatter S, Schocklmann H, Schreiber S, von Seydewitz CU, Steinhoff J, Stracke S, Suerbaum S, van de Loo A, Vischedyk M, Weissenborn K, Wellhoner P, Wiesner M, Zeissig S, Buning J, Schiffer M, Kuehbacher T (2012) Validation of treatment strategies for enterohaemorrhagic Escherichia coli O104:H4 induced haemolytic uraemic syndrome: case-control study. BMJ 345:e4565CrossRefPubMedPubMedCentral

go back to reference Wurzner R, Riedl M, Rosales A, Orth-Holler D (2014) Treatment of enterohemorrhagic Escherichia coli-induced hemolytic uremic syndrome (eHUS). Semin Thromb Hemost 40:508–516CrossRefPubMed Wurzner R, Riedl M, Rosales A, Orth-Holler D (2014) Treatment of enterohemorrhagic Escherichia coli-induced hemolytic uremic syndrome (eHUS). Semin Thromb Hemost 40:508–516CrossRefPubMed

go back to reference Loos S, Ahlenstiel T, Kranz B, Staude H, Pape L, Hartel C, Vester U, Buchtala L, Benz K, Hoppe B, Beringer O, Krause M, Muller D, Pohl M, Lemke J, Hillebrand G, Kreuzer M, Konig J, Wigger M, Konrad M, Haffner D, Oh J, Kemper MJ (2012) An outbreak of Shiga toxin-producing Escherichia coli O104:H4 hemolytic uremic syndrome in Germany: presentation and short-term outcome in children. Clin Infect Dis 55:753–759CrossRefPubMed Loos S, Ahlenstiel T, Kranz B, Staude H, Pape L, Hartel C, Vester U, Buchtala L, Benz K, Hoppe B, Beringer O, Krause M, Muller D, Pohl M, Lemke J, Hillebrand G, Kreuzer M, Konig J, Wigger M, Konrad M, Haffner D, Oh J, Kemper MJ (2012) An outbreak of Shiga toxin-producing Escherichia coli O104:H4 hemolytic uremic syndrome in Germany: presentation and short-term outcome in children. Clin Infect Dis 55:753–759CrossRefPubMed

go back to reference Pape L, Hartmann H, Bange FC, Suerbaum S, Bueltmann E, Ahlenstiel-Grunow T (2015) Eculizumab in typical hemolytic uremic syndrome (HUS) with neurological involvement. Medicine (Baltimore) 94:e1000CrossRefPubMed Pape L, Hartmann H, Bange FC, Suerbaum S, Bueltmann E, Ahlenstiel-Grunow T (2015) Eculizumab in typical hemolytic uremic syndrome (HUS) with neurological involvement. Medicine (Baltimore) 94:e1000CrossRefPubMed

go back to reference Harambat JDC,C, Godron A, Bordes CD,Y, Llanas B (2013) Eculizumab in shigatoxin associated hemolytic uremic syndrome: a single center matched-cohort study. Pediatr Nephrol 28:1347–1378CrossRef Harambat JDC,C, Godron A, Bordes CD,Y, Llanas B (2013) Eculizumab in shigatoxin associated hemolytic uremic syndrome: a single center matched-cohort study. Pediatr Nephrol 28:1347–1378CrossRef

go back to reference Percheron L, Gramada R, Tellier S, Salomon R, Harambat J, Llanas B, Fila M, Allain-Launay E, Lapeyraque AL, Leroy V, Adra AL, Bérard E, Bourdat-Michel G, Chehade H, Eckart P, Merieau E, Piètrement C, Sellier-Leclerc AL, Frémeaux-Bacchi V, Dimeglio C, Garnier A (2018) Eculizumab treatment in severe pediatric STEC-HUS: a multicenter retrospective study. Pediatr Nephrol. https://doi.org/10.1007/s00467-018-3903-9 Percheron L, Gramada R, Tellier S, Salomon R, Harambat J, Llanas B, Fila M, Allain-Launay E, Lapeyraque AL, Leroy V, Adra AL, Bérard E, Bourdat-Michel G, Chehade H, Eckart P, Merieau E, Piètrement C, Sellier-Leclerc AL, Frémeaux-Bacchi V, Dimeglio C, Garnier A (2018) Eculizumab treatment in severe pediatric STEC-HUS: a multicenter retrospective study. Pediatr Nephrol. https://​doi.​org/​10.​1007/​s00467-018-3903-9

go back to reference Loirat C, Fakhouri F, Ariceta G, Besbas N, Bitzan M, Bjerre A, Coppo R, Emma F, Johnson S, Karpman D, Landau D, Langman CB, Lapeyraque AL, Licht C, Nester C, Pecoraro C, Riedl M, van de Kar NC, Van de Walle J, Vivarelli M, Fremeaux-Bacchi V (2016) An international consensus approach to the management of atypical hemolytic uremic syndrome in children. Pediatr Nephrol 31:15–39CrossRefPubMed Loirat C, Fakhouri F, Ariceta G, Besbas N, Bitzan M, Bjerre A, Coppo R, Emma F, Johnson S, Karpman D, Landau D, Langman CB, Lapeyraque AL, Licht C, Nester C, Pecoraro C, Riedl M, van de Kar NC, Van de Walle J, Vivarelli M, Fremeaux-Bacchi V (2016) An international consensus approach to the management of atypical hemolytic uremic syndrome in children. Pediatr Nephrol 31:15–39CrossRefPubMed

go back to reference Dundas S, Murphy J, Soutar RL, Jones GA, Hutchinson SJ, Todd WT (1999) Effectiveness of therapeutic plasma exchange in the 1996 Lanarkshire Escherichia coli O157:H7 outbreak. Lancet 354:1327–1330CrossRefPubMed Dundas S, Murphy J, Soutar RL, Jones GA, Hutchinson SJ, Todd WT (1999) Effectiveness of therapeutic plasma exchange in the 1996 Lanarkshire Escherichia coli O157:H7 outbreak. Lancet 354:1327–1330CrossRefPubMed